Department of Biomedical Sciences, Ohio University Heritage College of Osteopathic Medicine, Athens, OH, 45701, USA.
Edison Biotechnology Institute, Konneker Research Labs, Ohio University, Athens, OH, USA.
Rev Endocr Metab Disord. 2021 Mar;22(1):17-29. doi: 10.1007/s11154-020-09600-6. Epub 2020 Oct 10.
Growth hormone (GH) induces pleiotropic effects on growth and metabolism via binding and subsequent activation of the growth hormone receptor (GHR) and its downstream signaling pathways. Growth hormone insensitivity (GHI) describes a group of disorders in which there is resistance to the action of GH and resultant insulin-like growth factor I (IGF-I) deficiency. GHI is commonly due to genetic disorders of the GH receptor causing GH receptor deficiency (e.g. Laron Syndrome (LS)), decreased activation of GHR, or defects in post-receptor signaling molecules. Genetically altered mouse lines have been invaluable to better understand the physiological impact of GHI due to the ability to do invasive and longitudinal measures of metabolism, growth, and health on a whole animal or in individual tissues/cells. In the current review, the phenotype of mouse lines with GHI will be reviewed. Mouse lines to be discussed include: 1) GHR-/- mice with a gene disruption in the GHR that results in no functional GHR throughout life, also referred to as the Laron mouse, 2) mice with temporal loss of GHR (aGHRKO) starting at 6 weeks of age, 3) mice transgenic for a GHR antagonist (GHA mice), 4) mice with GHI in select tissues or cells generated via Cre-lox or related technology, and 5) assorted mice with defects in post-receptor signaling molecules. Collectively, these mouse lines have revealed an intriguing role of GH action in health, disease, and aging.
生长激素 (GH) 通过与生长激素受体 (GHR) 及其下游信号通路结合并随后激活,对生长和代谢产生多效性影响。生长激素不敏感 (GHI) 描述了一组疾病,其中存在对 GH 作用的抵抗以及由此产生的胰岛素样生长因子 I (IGF-I) 缺乏。GHI 通常是由于 GH 受体的遗传障碍导致 GH 受体缺乏(例如 Laron 综合征 (LS))、GHR 激活减少或受体后信号分子缺陷引起的。由于能够对整个动物或单个组织/细胞进行代谢、生长和健康的侵入性和纵向测量,基因改变的小鼠品系对于更好地理解 GHI 的生理影响非常有价值。在当前的综述中,将回顾具有 GHI 的小鼠品系的表型。将讨论的小鼠品系包括:1) GHR-/- 小鼠,其 GHR 中的基因缺失导致一生中没有功能性 GHR,也称为 Laron 小鼠,2) 从 6 周龄开始暂时丧失 GHR (aGHRKO) 的小鼠,3) 转染 GH 拮抗剂的小鼠 (GHA 小鼠),4) 通过 Cre-lox 或相关技术在特定组织或细胞中产生 GHI 的小鼠,以及 5) 受体后信号分子缺陷的各种小鼠。这些小鼠品系共同揭示了 GH 作用在健康、疾病和衰老中的有趣作用。